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Prune belly syndrome

Disease definition

A rare lower urinary tract obstruction (LUTO) characterized by varying degrees of an enlarged urinary bladder, dilated ureters, hydronephrosis, and poorly contractile and disorganized detrusor and ureteral smooth muscle, in association with hypoplastic or absent midline abdominal skeletal musculature, and bilaterally undescended testes in males.

ORPHA:2970

Classification level: Disorder
  • Synonym(s):
    • Abdominal muscle deficiency syndrome
    • Eagle-Barret syndrome
    • Obrinsky syndrome
    • Triad syndrome
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant or X-linked recessive or Not applicable 
  • Age of onset: Antenatal, Neonatal
  • ICD-10: Q79.4
  • OMIM: 100100
  • UMLS: C0033770  C0265363
  • MeSH: C536477  D011535
  • GARD: 7479
  • MedDRA: 10051025

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The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.